Ayşe Topcu AKDUMAN, Kemal ÖZERKAN, İlkay ORAL

Wilson Hastalığı Olan Gebelerin Obstetrik Sonuçları

Amaç: Wilson Hastalığı bulunan annelerin gebeliğine ait obstetrik sonuçları sunmak.Gereç ve Yöntemler: Kliniğimizde takip edilmiş olan 4 gebe kadının geçirmiş olduğu toplam 6 gebeliğe ait kayıtlar retrospektif olarak tarandı. Bu gebelerin özellikleri, gebeliklerinin klinik seyirleri, gebeliklerinde uygulanan tedavi yöntemleri ve fetal-maternal sonuçlar literatür eşliğinde değerlendirildi.Bulgular: Gebelerin medyan yaşı ve doğum anındaki medyan gebelik haftası sırasıyla 33,5 yıl ve 19 hafta olarak saptandı. Üç gebelik 1. trimesterde, bir gebelik 2. trimesterde ve iki gebelik de 3. trimesterde sonlandırıldı. Sonlandırılma sebepleri ablasyo plasenta, akut fetal distres, missed abortus ve terapötik abortus olarak gözlendi.Tartışma ve Sonuç: Wilson hastalığı biliyer bakır atılım bozukluğu sonucu özellikle karaciğer, beyin, böbrek ve korneada toksik miktarda bakır birikmesi ile karakterize otozomal resesif genetik bir hastalıktır. İnfertilite ve düşüklere neden olmaktadır. Wilson hastalığı gebelikte komplikasyonlarla seyredebilen bir hastalıktır. Maternal ve fetal komplikasyonları en aza indirmek için hastalar tedavi ve takipleri açısından yakın gözlem altında bulundurulmalıdır.

Obstetric Outcomes in Pregnant Women with Wilson's Disease

Aim: We aimed to present the obstetric outcomes of pregnancies of women with Wilson's disease.Materials and Methods: Clinical records related to the six pregnancies experienced by four women with Wilson's disease who were followed at our clinic were reviewed retrospectively. The features of these pregnant women, the clinical course of the pregnancies, the treatment methods applied, and the fetal and maternal outcomes were investigated in the light of relevant literature. Results: The median age of the pregnant women and the median gestational week at delivery were 33.5 years and 19 weeks , respectively. Three pregnancies were terminated in the first trimester, one pregnancy was terminated in the second trimester and 3 pregnancies were terminated in the third trimester. Ablatio placentae, acute fetal distress, missed abortion, and therapeutic abortion were observed as the cause of termination.Discussion and Conclusion: Wilson's disease is an autosomal recessive genetic disorder characterized by the accumulation of toxic amounts of copper, particularly in the liver, brain, kidneys and cornea, as a result of defective biliary copper excretion. It causes miscarriages and infertility. Patients should be strictly monitored and receive appropriate treatment to minimize the maternal and fetal complications.

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